To investigate the efficacy of autophagy enhancement for therapy of argininosuccinic aciduria (ASA), the second most frequent UCD (Baruteau et al, 2019a), we investigated TB‐1 treatment in the hypomorphic murine model of argininosuccinate lyase (ASL) deficiency (AslNeo/Neo) that expresses approximately 16% of residual enzyme activity and recapitulates the main biochemical and clinical abnormalities of ASA patients (Erez et al, 2011; Nagamani et al, 2012; Baruteau et al, 2018; Burrage et al, 2020). This evidence concerns the gene ASL and urea cycle disorder.